Bullous systemic lupus erythematosus and lupus nephritis in a young girl

Bullous systemic lupus erythematosus [BSLE] is an autoimmune blistering disease occurring in patients with systemic lupus erythematosus [SLE]. It is a rare disease, especially in children. A 14-year-old girl initially presented with fatigue, generalized vesiculobullous skin lesions, and ulcers over the hard palate and oral mucosa. Clinical investigations revealed hematuria and proteinuria, a high erythrocyte sedimentation rate and titer of antinuclear antibody, and anti-double-stranded DNA. Skin biopsy findings were suggestive of BSLE. A renal biopsy confirmed the features of class V lupus nephritis. Based on the clinical features and investigations, a diagnosis of BSLE with nephritis was made. She received methylprednisolone pulse therapy and hydroxychloroquine; however, it did not alleviate the vesiculobullous eruption, so treatment with dapsone started and resulted in the dramatic disappearance of the lesions. Interruption of dapsone due to hemolysis did not aggravate the bullous disease. During follow-up, she had multiple flare-ups of disease and nephritis without rebound of bullous lesions. BSLE is a rare presentation of SLE in children. Differentiating it from other skin bullous diseases and SLE with blister is important for the correct management. The unusual presentation of this disease may delay the diagnosis and therefore requires a high index of clinical suspicion

Efficacy of co-administration of garlic extract and metformin for prevention of gentamycine-renal toxicity in wistar rats: a biochemical study

Gentamicin [GM] nephrotoxicity has been related to oxidative stress. Garlic and metformin [MF] have anti-oxadant activity and therefore, this study was aimed to evaluate the preventive and curative effects of garlic, MF and their combination on GM indeced tubular toxicity in Wistar rats. In a pre-clinical study, 70 male Wistar rats were randomly designated into 7 groups of 10 and treated as follows: Group 1: Received saline for 20 days. Group 2: Were injected 100 mg/kg/d of GM intraperitoneally [ip], for 10 days and saline for 10 more days. Group 3: Received GM for 10 days then 20 mg/kg garlic ip for the next 10 days. Group 4: Received GM for 10 days and MF [100 mg/kg] orally for the next 10 days. Group 5: Received GM for 10 days and a combination of MF and garlic for the next 10 days [100 and 20 mg/kg, respectively]. Group 6: The same as group 5but with half-doses of MF and Garlic. Group 7: Received GM for 10 days together with a combination ofMF and garlic. On 20th day of the experiment the serum blood urea nitrogen [BUN] and creatinine [Cr] were measured and compared in different groups. GM injection significantly increased the serum BUN and Cr [P < 0.05]. Administration of MF, garlic or their combination with or after injection of GM [high doses] could atenuate BUN and Cr. The results indicate that MF and garlic or their combination have curative and protective activity against GM nephrotoxicity

Association of neutrophil gelatinase associated lipocalin and cystatin c with kidney function in children with nephrotic syndrome

Nephrotic syndrome [NS] is a major clinical concern in human health, especially in children. Despite of the etiology, the prediction of remission in different treatment regimens based on suitable biomarkers is under development. The goal of this evaluation was the demonstration of correlation between serum level of Neutrophil gelatinase associated lipocalin [NGAL] and cystatin C with kidney function in patients with NS. During the period between September 2008 and December 2011, 52 patients admitted to St. Al Zahra University Hospital were selected for evaluation. The measured parameters consisted of NGAL, cystatin C, creatinine, albumin, blood urea nitrogen, urine protein, glomerular filtration rate. Demographic data were collected and considered in comparisons. Comparison between variables and their correlations were examined. Means of serum NGAL and cystatin C were significantly higher in case than the control group, P < 0.05. The mean of serum NGAL in patients without remission and who achieved remission were 23.09 [standard deviation [SD] +/- 10.11] and 36.26 [SD +/- 20.10] ng/ml respectively; P < 0.05. Serum NGAL levels had a correlation with the following factors: Systolic blood pressure, diastolic blood pressure [DBP], cystatin C, remission. Linear regression analysis showed a significant correlation between cystatin C and systolic and DBP. Based on the results, serum NGAL can be used as a prognostic marker for remission. In addition, NGAL and cystatin C are biomarkers of kidney injury in NS

Association of proteinuria with various clinical findings and morphologic variables of oxford classification in immunoglobulin a nephropathy patients

Immunoglobulin A nephropathy [IgAN] with nephrotic syndrome is an uncommon form of IgAN. Clinical and morphological characteristics of proteinuria in IgAN, especially when is in nephrotic range have not yet been fully examined. This study was aimed to correlate morphologic variables of the Oxford classification, and various clinical data with proteinuria in IgAN patients. We also aimed to demonstrate the significance of prevention of proteinuria as one of the important factors in progression of this disease. In an observational study conducted on IgAN patients, total of 114 biopsies were entered in the study. IgAN was diagnosed by light and immunofluorescence study. Of 114 patients 70.2% were male. Mean age of patients was 37.7 +/- 13.6 years. The mean of proteinuria was 1742 +/- 1324 mg/day. Also mean of serum creatinine [Cr] was 1.6 +/- 1.5 mg/dL. Of 114 patients, 11[9.6%] had nephrotic range proteinuria. In this study, there was a positive correlation between proteinuria and serum Cr, peri-glomerular fibrosis or interstitial fibrosis. There was a positive association between proteinuria and totally sclerotic glomeruli too. There was also a positive association between the amount of fibrous crescents and the level of proteinuria. Nephrotic proteinuria could just be seen in male patients. Also, nephrotic syndrome had a positive association with the number of crescents. Our findings firstly support the prognostic value of crescent due to its association with proteinuria and secondly imply the importance of treatment of proteinuria to prevent progression of IgAN

Oxford-MEST classification in IgA nephropathy patients: a report from Iran

There is a limited knowledge about the morphological features of IgA nephropathy [IgAN]in the middle east region. The objective of this study was to evaluate the spectrum of histopathological findings in IgAN patients at our laboratory. At this work, an observational study reported which was conducted on IgAN patients using the Oxford-MEST classification system. In this survey, of 102 patients 71.6% were male. The mean age of the patients was 37.7 +/- 13.6 years. Morphologic variables of MEST classification was as follows; M1: 90.2%, E: 32%, S: 67% also, T in grads I and II were in 30% and 19% respectively, while 51% were in grade zero. A significant difference was observed in segmental glomerulosclerosis [P=0.003] and interstitial fibrosis/tubular atrophy frequency distribution [P=0.045], between males and females. Furthermore, it was found that mesangial hypercellularity was more prevalent in yonger patients. Moreover, there was a significant correlation between serum creatinine and crescents [P<0.001]. There was also significant correlation of serum creatinine with segmental glomerulosclerosis [P<0.001]. Higher prevalence of segmental glomerulosclerosis and interstitial fibrosis/tubular atrophy, as the two of, four variables of Oxford-MEST classification of IgAN in male patients further attests that male gender is a risk factor in this disease. In this study the significant correlation between serum creatinine and crescent was in an agreement with previous studies and suggests for the probable accomodation of extracapillary proliferation as a new variable in MEST system